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Autonomic failure is a disorder of noradrenergic neurotransmission in which postganglionic sympathetic neurons do not release norepinephrine appropriately. This defect in norepinephrine release can be caused by a degenerative process affecting central (preganglionic) autonomic pathways (multiple system atrophy), disorders of postganglionic autonomic neurons (pure autonomic failure and peripheral autonomic neuropathies), and very rarely, by an enzymatic defect in catecholamine synthesis (dopamine beta hydroxylase deficiency). A precursor of norepinephrine that could be taken up by sympathetic neurons, converted to norepinephrine, and released could potentially treat autonomic failure. This hypothesis was tested 10 years ago by Biaggioni and Robertson [1] and Man in't Veldt et al. [2] These investigators showed that administration of the artificial aminoacid 3,4-dihydroxyphenyl serine (DOPS) dramatically improved orthostatic hypotension in autonomic failure caused by deficiency of the enzyme dopamine beta hydroxylase (the enzyme involved in the conversion of dopamine to norepinephrine). Orthostatic hypotension was relieved because after administration, DOPS is decarboxylated in the body directly to norepinephrine by aromatic amino acid decarboxylase in one step, thus bypassing dopamine beta hydroxylase.
Most forms of autonomic failure, however, are due to degeneration of pre- or postganglionic autonomic neurons rather than discrete enzymatic defects. Could treatment …
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